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Name the common syndromes that masquerade as anterior and posterior uveitis and their diagnostic tests. 5+5 PAPER III DNB question Papers 2011 (June)
Masquerade Syndrome
Group of disorders simulating/mimic Ant. Or Post. Uveitis in their C/F but are having entirely diff. etiopathogenesis being usually neoplastic or occasionally ischaemic
1967 :Theodore : used the term "Masquerade Syndrome" in ophthalmology to describe a case of conjunctival carcinoma that manifested as chronic conjunctivitis Many are malignant; prompt dx needed. Uveitis Masquerade accounts for 5 % of patients with uveitis in a tertiary care centre
When to suspect ?
Masquerade Syndromes
Anterior Segment
Posterior Segment
Masquerade syndromes
Lymphoid malignancies
Nonneoplastic
i. Lymphoma ii. Leukemia i.Uveal melanoma i. Choroidal metastasis ii. Paraneoplastic syndromes
Nonlymphoid malignancies
a. Chronic peripheral retinal detachment b. Retinitis pigmentosa c. Intraocular foreign body d. Amyloidosis e. Vitreous hemorrhage f. Retinal vascular disease
i. Ocular ischemic syndrome ii. Hypertensive retinopathy iii. Diabetic retinopathy iv. Vascular occlusion
g. Coats diseasea
Synonyms : large B-cell lymphoma, reticulum cell sarcoma. The most common neoplastic masquerader(68%) NHL involving eye is a rare cond. Which mimics ch. Uveitis or post. Uveitis Ocular lymphomas occur in both primary & secondary forms Site of origin of PCNSL is unknown Yet are able to grow only in these two immunologically sequestered locations (CNS or eye)
CLASSIFICATION of Lymphoma
Intraocular Lymphoma
Intraocular lymphoma can masquerade as a chronic infection and may be a sign of CNS malignancy. Etiopathogenesis
Demographics
Vitritis: impede visualization of the fundus. Large solid multifocal subretinal infiltrates Occasionally coalescence of sub-RPE deposits may form a ring encircling the equator Other features include
retinal vasculitis, vascular occlusion, exudative retinal detachment and optic atrophy.
Lack of CMO is an important diagnostic clue, since in true uveitis significant vitritis is almost always accompanied by CMO.
Anterior segment of the eye of a patient with biopsy-prove intraocular large-cell lymphoma demonstrating corneal keratitic precipitates
Slit-lamp biomicroscopy examination revealing sheets of vitreous cells characteristic of intraocular lymphoma
Fundus photograph of patient with intraocular lymphoma showing retinal infiltrates and hemorrhage mimicking Cytomegalovirus retinitis.
PATHOLOGY
Thickening of detatched retina ( Pizza-Pie appearance ) Subretinal pigment elevation Vitreous infiltration FA shows blockage with a granular characteristic, due to the presence of subRPE accumulation of lymphomatous cells (leopard skin spots).
Intraocular Lymphoma
Systemic Findings
Behavioral changes hemiparesis Ataxia MRI. Lumbar puncture. Vitreous biopsy for cytology, flow cytometry, interleukin-6 and -10, immunoglobulin heavy chain gene rearrangement. Radiation therapy. Chemotherapy (intrathecal methotrexate or cytosine arabinoside [ara-C]) . Biologic agents involving specific anti-B cell monoclonal antibodies (such as rituximab
Special Tests
Initial workup may be negative, but maintain a high clinical suspicion in uveitis refractory to treatment.
SYSTEMIC LYMPHOMA
Epidemiology
Rare intraocular involvement, older adults Vitreitis, retinal vasculitis, necrotizing retinitis, diffuse choroiditis, focal uveal masses, AU, hypopyon Lymphadenopathy, involvement of retroperitoneum, paranasal sinuses, orbits, meninges, bone marrow
Ocular Manifestations:
Systemic Associations:
Comments:
ocular involvement may be initial sign average longevity of 31 months following ocular diagnosis
RETINOBLASTOMA
Most common primary, malignant, intraocular tumour of childhood (1:20,000) No sexual predilection Presents before age of 3 years (average 3 months) Heritable (40%) or non-heritable (60%) Predisposing gene (RPE 1) on 13q14 AU(13%), vitreitis, shifting white hypopyon
Presentations of retinoblastoma
Leukocoria - 60%
Early endophyliticretinoblastoma
Placoid lesion
Vitreous seedings
Exophytic retinoblastoma
Pseudohypopyon was an unusual first sign of retinoblastoma in a 6-year-old girl without a positive family history
RETINOBLASTOMA
Investigations
Ultrasound, aqueous tap USG, CT, MRI, Bonemarrow aspiration, lumbar puncture Calcification(75%) on ultrasonography and CT scan may help to differentiate retinoblastoma from various forms of childhood uveitis (toxoplasmosis, toxocariasis, cysticercosis, and pars planitis)
CT diagnosis of retinoblastoma
Calcification
2. Medium tumours
3. Large tumours
Chemotherapy followed by local treatment Enucleation
4. Extraocular extension
External beam radiotherapy
5. Metastatic disease
Chemotherapy
10% of uveal melanomas C/F 6 th decade Dilated episcleral vessels in same quadrants (Sentinel vessels ) Astigmatism, Subluxation, Cataract RD Necrotic tumors may elicit an intense inflammatory response associated with seeding of tumor cells into the vitreous cavity and anterior segment
Prognosis - guarded
Sentinel vessels
Extraocular extension
Uveal melanoma
Epidemiology:
4.9% of 450 enucleation specimens had ocular inflammation initially Episcleritis, AU, PU, endophthalmitis, panophthalmitis May have features of metastatic melanoma
Ocular Manifestations:
Systemic Associations:
Choroidal melanoma
Most common uveal melanoma 80% of cases Presentation - sixth decade Prognosis - usually good
Choroidal folds
Surface orange pigment (lipofuscin) is common Mushroom-shaped if breaks through Bruchs membrane
LEUKAEMIAS :INTRODUCTION
ALL- Children are affected AML- Worse prognosis,Adults more affected Chronic leukaemias Elderly affected
2875% with acute leukemia less with chronic
Ocular findings in
SCH Iritis & Hypopyon Diffuse & Nodular iris thickening Hyphaema Orbital involvement Optic neuropathy
Anterior segment infiltration in acute myeloid leukemia (AML) presenting as hypopyon uveitis
Relapsing acute myeloid leukemia presenting as hypopyon uveitis Sapna P Hegde1, Atul T Ursekar2, Ajay A Chitale3
LEUKAEMIAS Retinopathy
Venous tortuosity & dilatation Flame shaped Hge & ROTH SPOTS Cotton wool spots Peripheral retinal neovascularisation LEOPARD SPOT RETINA
Diagnostic Tests
JUVENILE XANTHOGRANULOMA
Vascular Tm. Affecting skin & eye Important cause spontaneous hyphaema & seconary glaucoma Epidemiology:
Ocular Manifestations:
Metastatic tumors
Epidemiology :
Most common intraocular malignancy in adults Usually bilateral, multifocal, plateau-shaped, yellow posterior segment lesions with SRF; anterior segment: AU, iris nodules, NVI Lung and breast carcinoma most common primary sites
Ocular Manifestations:
Systemic Associations :
May produce a cellular reaction in the anterior or posterior chamber as well as PS. Diagnostic Tests
Ophthalmoscopy Ultrasound
Retained intraocular foreign body associated with trauma may cause persistent anterior and/ or posterior segment inflammation. Diagnostic Tests:
X-ray Ultrasound CT scan( should demonstrate the abnormality)
Retinitis pigmentosa
May present with vitreous cells and posterior subcapsular cataract The bone spicule pigment deposition in the retina, attenuated retinal vessels, mottling and atrophy of the RPE, and waxy pallor of the optic nerve help to distinguish this disease from other disorders. The diagnosis can be confirmed with an extinguished electroretinogram and ring scotoma on visual field testing.
Kruckenbergs spindle in a patient with pigment dispersion syndrome that masqueraded as anterior uveitis.
Diagnostic Tests:
IVFA Carotid Doppler
90%
Gradual visual loss Aching pain Prolonged recovery following bright light exposure
Narrowed arteries Dilated, non-tortuous veins Mid-peripheral retinal hemorrhages, microaneurysms Neovascularization
Delayed choroidal filling (60%) Delayed arteriovenous transit (95%) Vascular staining (85%)
What is the 5-year mortality rate and what is the most common cause of death?
Coats disease
Retinal and subretinal hard exudation Overlying vascular dilatation and tortuosity May benefit from photocoagulation
Slow progresssion of exudation Exudative retinal detachment and retrolental mass Severe visual loss Treatment not beneficial
FA of Coats disease
CONCLUSION
It is clear that a number of entities can masquerade as idiopathic uveitis, and in the absence of a correct diagnosis inappropriate therapy may be prescribed. The diagnosis of a masquerade syndrome should therefore be considered in the evaluation of all patients with undiagnosed inflammatory eye disease